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Y2 Rheumatology > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

1
Q

What are Connective Tissue Diseases?

A 
Multisystem disorders that can cause organ pathology
SLE
Sjogrens
Systemic Sclerosis
Mixed Connective Tissue Disease
Anti-phospholipid Syndrome
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2
Q

What is the proposed pathogenesis of SLE?

A

Defect in apoptosis –> increased cell death
Defective clearance of apoptotic cell debris –> persistence of antigen & immune complex production
Circulating immune complexes with antigens in various tissues

in small BVs: complement activation & inflammation
BMs of skin & kidneys

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3
Q

Environmental & genetic basis of SLE

A

UK : &S: Black&raquo_space; white (however disease rarely reported in Africa)
40% concordance in MZ

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4
Q

F:M of SLE

A

F:M is 11:1 in childbearing years

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5
Q

onset age of SLE

A

after puberty - 20-30s

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6
Q

% of SLE cases in women

A

90%

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7
Q

Constitutional signs & symptoms of SLE

A

fever
fatigue
weight loss

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8
Q

Other typical signs & symptoms of SLE (there’s way too many)

A 
arthralgia, myaliga, AVN maybe
malar rash, photosensitivity
discoid lupus, Raynaud's
lupus nephritis
lung
haematological
neuropsychiatric
cardiac
GI
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9
Q

Investigation of SLE

A

no one diagnostic test
FBC may show: anaemia, leucopenia, thrombocytopenia
urinalysis: look for glomerulonephritis
imaging: CT, MRI, Echo (organ involvement)
serology

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10
Q

Serology for SLE

A

ANA: high sensitivity, low specificity
Anti-dsDNA: varies with disease activity (so may not be super sensitive), high specificity
Anti-Sm: low sensitivity, high specificity
C3/4: sensitivity low when disease is active

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11
Q

Specific disease markers for SLE

A

Anti-dsDNA, Anti-Sm

ANA not specific

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12
Q

Sensitive disease markers for SLE

A

ANA very sensitive (not specific though)
Anti-dsDNA varies with disease activity
(Anti-Sm low sensitivity)

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13
Q

Which marker is used for disease activity in SLE

A

Anti-dsDNA

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14
Q

Which marker is used for disease activity in SLE

A

Anti-dsDNA

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15
Q

SLICC classification criteria for SLE

A

4 or more criteria with at least 1 clinical & 1 laboratory
or biopsy-proven lupus nephritis with positive ANA / Anti-DNA

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16
Q

Clinical Criteria for SLE (11)

A 
Acute Cutaneous Lupus
Chronic Cutaneous Lupus
Oral / Nasal Ulcers
Non-scarring alopecia
Arthritis
Serositis
Renal
Neurologic
Haemolytic anaemia
Leukopenia
Thrombocytopenia
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17
Q

Immunologic Criteria for SLE

A 
ANA
Anti-DNA
Anti-Sm
Antipohspholipid Ab
Low complement (C3,4)
Direct Coomb's test
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18
Q

Treatment of SLE of there is skin disease & arthralgia

A

hydroxychloroquine
topical steroids
NSAIDs

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19
Q

Treatment of SLE if there is inflammatory arthritis/ evidence of organ involvement (pericardial disease, ILD)

A

immunosuppression: azathioprine /myocephanolate mofetil

Corticosteroids at moderate doses for short periods

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20
Q

Treatment of SLE if there is severe organ disease (lupus nephritis / CNS lupus)

A

IV steroids, cyclophosphamide

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21
Q

Treatment of unresponsive SLE

A

IV immunoglobulin, rituximab

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22
Q

Monitoring of SLE

A

according to symptoms
check regularly: Anti-dsDNA, C3/4
urinalyiss- check for glomerulonephritis
evaluate & manage CV risk factors - BP, cholesterol

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23
Q

What is the pathogenesis of Sjogrens Syndrome?

A

Autoimmune - lymphocytic infiltrates in exocrine organs

24
Q

Aetiology of Sjogrens Syndrome:

A

Primary

Secondary to other AI - RA, SLE

25
Q

Symptoms of Sjogrens Syndrome

A

sicca symptoms: dry eye & mouth
other symptoms: arthralgia, fatigue, vaginal dryness, parotid gland swelling
may also occur: peripheral neuropathy & ILD, increased risk of lymphoma

26
Q

Diagnosis of Sjogrens Syndrome

A

4 of the following:
occular symptoms:
-dry eyes for > 3 months
-foreign-body sensation
-use of tear substitutes > 3x/day
oral symptoms:
-dry mouth
-recurrently swollen salivary glands
-frequent use of liquids to aid swallowing
ocular signs
-Schimer test without anaesthesia (<5mm in 5min)
-positive vital dye stainint result
oral signs
-abnormal salivary scintigraphy findings
-abnormal parotid gland scintigraphy findings
-abnormal sialometry findings
-unstimulated salivary flow <1.5 mL in 15 min
positive minor salivary gland biopsy findings
positive anti-SSA/anti-SSB (Ro or LA)

27
Q

Serology in Sjogrens Syndrome

A

Anti-SSA/Anti-SSB (Ro / LA)

28
Q

Oral Symptoms & Signs of Sjogrens Syndrome

A 
Symptoms:
-dry mouth
-recurrent swollen salivary glands
-frequent use of liquids to aid swallowing
Signs:
-abnormal salivary scintigraphy findings
-abnormal parotid scintigraphy findings
-abnormal sialometry findings 
-unstimulated salivary flow <1.5 mL in 15 min
29
Q

Oral Symptoms & Signs of Sjogrens Syndrome

A 
Symptoms:
-dry mouth
-recurrent swollen salivary glands
-frequent use of liquids to aid swallowing
Signs:
-abnormal salivary scintigraphy findings
-abnormal parotid scintigraphy findings
-abnormal sialometry findings 
-unstimulated salivary flow <1.5 mL in 15 min
30
Q

What is the Pathogenesis of Systemic Sclerosis

A

Excessive Collagen Deposition leading to skin & internal organ changes
Vasomotor disturbances (Raynaud’s)
Fibrosis
–> Atrophy of skin & subcutaenous tissue
3 phases of cutaneous symptoms: odematous, indurative, atrophic

31
Q

3 phases of cutaneous symotoms of SSC

A

odematous
indurative
atrophic

32
Q

What leads to death in SSC?

A

renal & lung changes

pulmonary hypertension leads to 12% of SSC-related deaths

33
Q

Signs & Symptoms of SSC

A 
Raynaud's phenomenon
Thickened &amp; Tight skin
-skin of face: pinching of the skin of nose (beaking)
-tightening of the skin around the mouth
Telangiectasia
Calcinosis - may be seen in digits
34
Q

Major & Minor Features of SSC

A 
Major: 
-centrally located skin sclerosis 
-arms, face, neck
Minor: 
-sclerodactyly &amp; atrophy of fingertips
-bilateral lung fibrosis
35
Q

Organ involvement in SSC

A

lung: pulmonary HTN, pulmonary fibrosis
accelerated HTN –>
renal crisis
gut: dysphagia, malabsorption, bacterial overgrowth of small bowel
MSK: inflammatory arthritis & myositis

36
Q

Limited SSC other name

A

CREST

37
Q

Limited SSC disease progression

A

skin involved confined to face, hands, forearms & feet

organ involvement occurs later

38
Q

Diffuse SSC disease progression

A

skin changes develop more rapidly, can involve the trunk

early significant organ involvement

39
Q

Limited SSC Ab

A

Anti- Centromere

40
Q

Diffuse SSC Ab

A

Anti-Scl-70

41
Q

Investigations of SSC

A 
Immunology
Organ screening regularly: 
-pulmonary function test, 
-echocardiogram, 
-monitor renal function
42
Q

Diagnosis of SSC

A

1 major & 2 minor features

43
Q

Major features of SSC

A

centrally located skin sclerosis

arms, face, neck

44
Q

Minor features of SSC

A

sclerodactyly and atrophy of the fingertips

bilateral lung fibrosis

45
Q

Treatment of SSC

A

Raynauds/digital ulcers: CCBs (Iloprost, Bosentan)
Renal involvement: ACEi
GI involvement: PPI for reflux
ILD: immunosuppression - cyclophosphamide

46
Q

Features & Symptoms of MCTD

A 
Raynauds
Arthralgia/Arthritis
Myositis
Sclerodactyly
Pulmonary HTN
ILD
47
Q

Serology for MCTD

A

anti-RNP Ab

48
Q

Monitoring of MCTD

A

Regular echocardiograms - risk of pulmonary htN

Pulmonary function test - screen for ILD

49
Q

Treatment of MCTD

A

Depends on presentation
Raynauds: CCBs (Iloprost, Bosentan)
Muscle/lung disease: immunosuppression

50
Q

What is the pathology behind Anti-Phospholipid Syndrome?

A

Recurrent venous/arterial thrombosis and/or fetal loss

51
Q

Associated diseases to Anti-Phospholipid Syndrome

A

SLE or other AI disorders

52
Q

Signs & Symptoms of Anti-Phospholipid Syndrome

A
  • Increased frequency of stroke/MI
  • Recurrent pulmonary emboli/thrombosis - pulmonary HTN
  • Catastrophic ACS (CAPS): multiorgan infarction over a period of days-weeks
  • Late spontaneous fetal loss (2nd or 3rd trimester)
  • Recurrent early fetal loss (10 weeks) also possible
  • Migraine
  • Livedo reticularis
53
Q

In Anti-Phospholipid Syndrome, what is stroke due to?

A

In situ thrombosis

Embolization origination from the valvular lesions of Libman-Sacks (Sterile) Endocarditis

54
Q

What is a common cutaneous finding in APS?

A

Livedo Reticularis

55
Q

Investigations of APS

A

Thrombocytopenia
Prolongation of APTT
Immunology

56
Q

Serology of APS

A

Lupus ANticoagulant
Anti-cardiolipin
Anti-beta 2 glycoprotein

57
Q

Treatment of APS

A

episode of thrombosis: Anti-coagulation
recurrent pregnancy loss: LMWH during pregnancy (Warfarin would be teratrogenic)
Positive Ab without thrombosis event: don’t require anticoagulation

Y2 Rheumatology (7 decks)

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